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Hypoplastic left heart syndrome

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https://www.readbyqxmd.com/read/28928623/hypoplastic-left-heart-syndrome-with-coronary-cameral-fistulas-echocardiographic-demonstration-of-coronary-artery-steal-and-successful-interventional-treatment
#1
Arshid Mir, Mary Niu, Merick Yamada, Ed Overholt
We report a case of a patient with hypoplastic left heart syndrome with significant coronary-cameral fistulas and exertional symptoms from coronary steal. Symptoms resolved following successful coil occlusion of his left ventricle.
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28924546/sirenomelia-associated-with-hypoplastic-left-heart-in-a-newborn
#2
H Turgut, R Ozdemir, I K Gokce, C Karakurt, A Karadag
Sirenomelia, also known as "mermaid malformation/syndrome," is a rare, serious congenital anomaly characterized by variable degrees of fusion of the lower limbs and associated with severe malformations of vertebral, genitourinary, cardiovascular system and single umbilical artery. The first pregnancy of a 25-year-old woman resulted in one twin born by Cesarian section at 32 weeks' gestation, who was referred to our hospital with cyanosis, a congenital anomaly and respiratory distress. On physical examination, there was no urogenital region and anal fissure and gender was indeterminate...
June 30, 2017: Balkan Journal of Medical Genetics: BJMG
https://www.readbyqxmd.com/read/28922269/extubation-failure-is-associated-with-increased-mortality-following-first-stage-single-ventricle-reconstruction-operation
#3
Thomas Scodellaro, Jane M McKenzie, Yves d'Udekem, Warwick Butt, Siva P Namachivayam
OBJECTIVES: To identify the prevalence, causes, risk factors, and outcomes associated with extubation failure following first stage single ventricle reconstruction surgery. DESIGN: Retrospective cohort analysis of neonates who underwent a first stage single ventricle reconstruction operation. Extubation failure was defined as endotracheal reintubation within 48 hours of first extubation attempt. SETTING: The Royal Children's Hospital, Melbourne...
September 15, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28921168/chromosomal-abnormalities-affect-the-surgical-outcome-in-infants-with-hypoplastic-left-heart-syndrome-a-large-cohort-analysis
#4
Dala Zakaria, Xinyu Tang, Rupal Bhakta, Nahed O ElHassan, Parthak Prodhan
Patients with hypoplastic left heart syndrome (HLHS) can have associated genetic abnormalities. This study evaluated the incidence of genetic abnormalities among infants with HLHS and the short-term outcomes of this population during the first hospitalization. This is a retrospective analysis of the multi-center Pediatric Heath Information System database of infants with HLHS who underwent Stage I Norwood, Hybrid, or heart transplant during their first hospitalization from 2004 through 2013. We compared clinical data between infants with and without genetic abnormality, among the three most common chromosomal abnormalities, and between survivors and non-survivors...
September 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28918207/the-association-between-cardiac-physiology-acquired-brain-injury-and-postnatal-brain-growth-in-critical-congenital-heart-disease
#5
Shabnam Peyvandi, Hosung Kim, Joanne Lau, A James Barkovich, Andrew Campbell, Steven Miller, Duan Xu, Patrick McQuillen
OBJECTIVE: To assess the trajectory of perioperative brain growth in relationship to cardiac diagnosis and acquired brain injuries. METHODS: This was a cohort study of term neonates with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). Subjects underwent magnetic resonance imaging of the brain pre- and postoperatively to determine the severity of brain injury and total and regional brain volumes by the use of automated morphometry...
August 24, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28904928/prenatal-sonographic-diagnosis-of-hypoplastic-left-heart-syndrome
#6
Raghu Teja Sadineni, B Santh Kumar, N B Chander, Durga Mahita Boppana
Hypoplastic left heart syndrome (HLHS) represents a variety of cardiac malformations that may result from errors in the early stages of cardiac development. HLHS includes a wide spectrum of cardiac malformations including hypoplasia of the left ventricle, ascending aorta, hypoplasia, or atresia of the aortic and mitral valves. Over the recent years, the improved resolution of advanced equipment with awareness and increased performance of second-trimester ultrasound examinations for the assessment of fetal anomalies have helped in understanding the spectrum and have expanded our knowledge of HLHS...
July 2017: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/28893257/preoperative-single-ventricle-function-determines-early-outcome-after-second-stage-palliation-of-single-ventricle-heart
#7
Jacek Pająk, Michał Buczyński, Piotr Stanek, Grzegorz Zalewski, Marek Wites, Lesław Szydłowski, Bogusław Mazurek, Lidia Tomkiewicz-Pająk
BACKGROUND: Second-stage palliation with hemi-Fontan or bidirectional Glenn procedures has improved the outcomes of patients treated for single-ventricle heart disease. The aim of this study was to retrospectively analyze risk factors for death after second-stage palliation of single-ventricle heart and to compare therapeutic results achieved with the hemi-Fontan and bidirectional Glenn procedures. MATERIAL AND METHODS: We analyzed 60 patients who had undergone second-stage palliation for single-ventricle heart...
September 11, 2017: Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/28889833/acute-kidney-injury-following-first-stage-palliation-in-hypoplastic-left-heart-syndrome-hybrid-versus-norwood-palliation
#8
Richard U Garcia, Girija Natarajan, Henry L Walters, Ralph E Delius, Sanjeev Aggarwal
OBJECTIVE: The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes. Design and patients This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889828/atrial-septal-defect-morphology-and-stenting-in-hypoplastic-left-heart-syndrome-after-hybrid-palliation
#9
Lilia Oreto, Giuseppe Mandraffino, Lucia Manuri, Michele B Saitta, Salvatore Agati, Concetta Zito, Fiore S Iorio, Scipione Carerj, Paolo Guccione
Aims The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect. Methods and results From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889813/unique-levoatriocardinal-veins-in-neonates-with-hypoplastic-left-heart-syndrome-and-intact-atrial-septum
#10
Chiaki Iida, Jun Muneuchi, Mamie Watanabe
We had two cases of neonates with hypoplastic left heart syndrome and intact atrial septum who had unique levoatriocardinal veins. Contrast-enhanced CT and angiography revealed that previously unknown communicating vessels ran from the top of the left atrium and drained into the right atrium. We emphasise that transcatheter atrial septostomy should be performed not through these communicating vessels but using the trans-septal approach in neonates with hypoplastic left heart syndrome and an intact atrial septum...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28884922/congenital-heart-defects-in-molecularly-proven-kabuki-syndrome-patients
#11
Maria Cristina Digilio, Maria Gnazzo, Francesca Lepri, Maria Lisa Dentici, Elisa Pisaneschi, Anwar Baban, Chiara Passarelli, Rossella Capolino, Adriano Angioni, Antonio Novelli, Bruno Marino, Bruno Dallapiccola
The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%)...
September 8, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28879411/serial-changes-in-anatomy-and-ventricular-function-on-dual-source-cardiac-computed-tomography-after-the-norwood-procedure-for-hypoplastic-left-heart-syndrome
#12
Hyun Woo Goo
BACKGROUND: Accurate evaluation of anatomy and ventricular function after the Norwood procedure in hypoplastic left heart syndrome is important for treatment planning and prognostication, but echocardiography and cardiac MRI have limitations. OBJECTIVE: To assess serial changes in anatomy and ventricular function on dual-source cardiac CT after the Norwood procedure for hypoplastic left heart syndrome. MATERIALS AND METHODS: In 14 consecutive patients with hypoplastic left heart syndrome, end-systolic and end-diastolic phase cardiac dual-source CT was performed before and early (average: 1 month) after the Norwood procedure, and repeated late (median: 4...
September 6, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28867155/the-boundaries-of-fetal-cardiac-intervention-expand-or-tighten
#13
REVIEW
Laura Gellis, Wayne Tworetzky
Fetal cardiac intervention (FCI) is a relatively new and continually evolving field, and, for select cardiac defects, offers the potential to alter the progression of the disease and improve outcomes. It is a procedure that requires a collaborative effort between maternal-fetal medicine, interventional cardiology and fetal echo/ultrasound specialists, as well as fetal and maternal anesthesiologists, nursing specialists, and social workers. This article reviews the most recently reported data and advances in FCI...
August 31, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28864444/hypoplastic-left-heart-syndrome-with-intact-or-restrictive-atrial-septum-a-report-from-the-international-fetal-cardiac-intervention-registry
#14
David W Jantzen, Anita J Moon-Grady, Shaine A Morris, Aimee K Armstrong, Christoph Berg, Joanna H Dangel, Carlen G Fifer, Michele Frommelt, Ulrich Gembruch, Ulrike Herberg, Edgar T Jaeggi, Eftichia V Kontopoulos, Audrey C Marshall, Owen Miller, Renate Oberhoffer, Dick Oepkes, Carlos A Pedra, Simone R Pedra, Fabio Peralta, Ruben A Quintero, Greg Ryan, Sarah K Gelehrter
Infants with hypoplastic left heart syndrome and an intact or highly restrictive atrial septum (HLHS-IAS) represent a high risk subset. Fetal cardiac intervention (FCI) for fetuses with HLHS-IAS has been reported in single-institution series as a therapy that may improve outcomes. This study utilizes the International Fetal Cardiac Intervention Registry (IFCIR) to examine fetal and maternal characteristics and pregnancy and neonatal outcome data for FCI in this population.
September 1, 2017: Circulation
https://www.readbyqxmd.com/read/28863042/the-bioengineered-cardiac-left-ventricle
#15
Nikita M Patel, Ravi K Birla
Left ventricle and aortic valve underdevelopment are presentations in the congenital cardiac condition hypoplastic left heart syndrome (HLHS); current clinical treatments involve right ventricle refunctionalization. Cardiac organoid models provide simplified open chambers engineered into a flow loop, to ameliorate ventricle-type function. Complete bioengineered ventricle development presents a significant advancement in cardiac organoids. This study provides the foundation for bioengineered complete ventricle (BECV) fabrication...
August 31, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28851719/surgical-and-catheter-based-reinterventions-are-common-in-long-term-survivors-of-the-fontan-operation
#16
Tacy E Downing, Kiona Y Allen, David J Goldberg, Lindsay S Rogers, Chitra Ravishankar, Jack Rychik, Stephanie Fuller, Lisa M Montenegro, James M Steven, Matthew J Gillespie, Jonathan J Rome, Thomas L Spray, Susan C Nicolson, J William Gaynor, Andrew C Glatz
BACKGROUND: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation. METHODS AND RESULTS: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%. By 20 years after Fontan, 65% of patients had experienced either surgical or transcatheter intervention. The median time to first reintervention was 9...
September 2017: Circulation. Cardiovascular Interventions
https://www.readbyqxmd.com/read/28838527/aortic-dissection-after-the-hybrid-stage-1-procedure-diagnosis-and-management-strategy
#17
Scott Hittinger, Varsha Bandisode, Shahryar Chowdhury, Minoo N Kavarana
Hybrid Norwood palliation is gaining acceptance as an alternative to open Norwood palliation in high-risk neonates with hypoplastic left heart syndrome. Aortic dissection after hybrid Norwood palliation is a rare but ominous complication that has not been reported.
September 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28834879/effect-of-acid-base-balance-on-postoperative-course-in-children-with-hypoplastic-left-heart-syndrome-after-the-modified-norwood-procedure
#18
Marcin Gładki, Tomasz Składzień, Rafał Żurek, Elżbieta Broniatowska, Elżbieta Wójcik, Janusz H Skalski
Hypoplastic left heart syndrome (HLHS) is a congenital heart defect that requires 3-stage cardiac surgical treatment and multidirectional specialist care. The condition of newborns in the first postoperative days following the modified Norwood procedure is characterized by considerable hemodynamic instability that may result in a sudden cardiac arrest. It is believed that the most important cause of hemodynamic instability is the fluctuations in redistribution between pulmonary and systemic blood flow.The paper analyzes the postoperative course in 40 neonates with HLHS following the modified Norwood procedure performed under deep hypothermic cardiopulmonary bypass hospitalized in Cardiac Surgical Intensive Care Unit (CSICU) in the years 2014-2015...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28832800/modified-hybrid-procedure-in-hypoplastic-left-heart-syndrome-initial-experience-of-a-center-in-northeastern-brazil
#19
Renato Max Faria, Juliana Torres Pacheco, Itamar Ribeiro de Oliveira, José Madson Vidal, Anilton Bezerra Rodrigues, Ana Luiza Lafeta Costa, Vinicius José da Silva Nina, Marcelo Matos Cascudo
Introduction: Although it only corresponds to 2.5% of congenital heart defects, hypoplastic left heart syndrome (HLHS) is responsible for more than 25% of cardiac deaths in the first week of life. Palliative surgery performed after the second week of life is considered an important risk factor in the treatment of HLHS. Objective: The aim of this study is to describe the initial experience of a medical center in Northeastern Brazil with a modified off-pump hybrid approach for palliation of HLHS...
May 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28831564/post-operative-outcomes-in-children-undergoing-fontan-palliation-in-a-regionalized-surgical-system
#20
Billie-Jean Martin, David B Ross, Mohammed Al Aklabi, Joyce Harder, John D Dyck, Ivan M Rebeyka
Evidence suggests that outcomes in pediatric cardiac surgery are improved by consolidating care into centers of excellence. Our objective was to determine if outcomes are equivalent in patients across a large regional referral base, or if patients from centers without on-site surgery are at a disadvantage. Since 1996, all pediatric cardiac surgery has been offered at one of two centers within the region assessed, with the majority being performed at Stollery Children's Hospital. All patients who underwent a Fontan between 1996 and 2016 were included...
August 22, 2017: Pediatric Cardiology
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